Pentosan polysulfate maculopathy: Keep an eye out for this masquerader
DOI:
https://doi.org/10.58931/cect.2022.1211Abstract
Pentosan polysulfate sodium (PPS) (Elmiron; Janssen Pharmaceuticals), a drug used to treat bladder pain and discomfort associated with interstitial cystitis (IC), has been linked to a distinctive vision-threatening maculopathy. As with the case of hydroxychloroquine maculopathy, it is worthwhile for the general ophthalmologist to be familiar with this potentially preventable condition. In this article, we briefly summarize the evidence supporting this association, review the clinical manifestations of PPS maculopathy, and provide some guidance regarding screening protocols.
References
Pearce WA, Chen R, Jain N. Pigmentary Maculopathy Associated with Chronic Exposure to Pentosan Polysulfate Sodium. Ophthalmology. Nov 2018;125(11):1793-1802. doi:10.1016/j.ophtha.2018.04.026 DOI: https://doi.org/10.1016/j.ophtha.2018.04.026
Interstitial Cystitis (Painful Bladder Syndrome). National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). July 2017. Available at https://www.niddk.nih.gov/health-information/urologic-diseases/interstitial-cystitis-painful-bladder-syndrome/definition-facts. Accessed 23 January 2022.
Nickel JC, Teichman JM, Gregoire M, Clark J, Downey J. Prevalence, diagnosis, characterization, and treatment of prostatitis, interstitial cystitis, and epididymitis in outpatient urological practice: the Canadian PIE Study. Urology. Nov 2005;66(5):935-40. doi:10.1016/j.urology.2005.05.007 DOI: https://doi.org/10.1016/j.urology.2005.05.007
Janssen Pharmaceuticals. Elmiron (pentosan polysulfate sodium) [package insert]. U.S. Food and Drug Administration. March 2021. Available at https://www.accessdata.fda.gov/drugsatfda_docs/label/2020/020193s014lbl.pdf. Accessed 5 January 2022.
Elmiron – intravescial instillation. Janssen Pharmaceuticals. 5 April 2021. Available at https://www.janssenmd.com/elmiron/dosage-and-administration/administration-routes/elmiron-intravesical-instillation?disclaimer&tid=171990374961d5dfcf3abf47.64211558. Accessed 5 January 2022.
Lindeke-Myers A, Hanif AM, Jain N. Pentosan polysulfate maculopathy. Surv Ophthalmol. Jan-Feb 2022;67(1):83-96. doi:10.1016/j.survophthal.2021.05.005 DOI: https://doi.org/10.1016/j.survophthal.2021.05.005
Hanif AM, Shah R, Yan J, et al. Strength of Association between Pentosan Polysulfate and a Novel Maculopathy. Ophthalmology. Oct 2019;126(10):1464-1466. doi:10.1016/j.ophtha.2019.04.024 DOI: https://doi.org/10.1016/j.ophtha.2019.04.024
Vora RA, Patel AP, Melles R. Prevalence of Maculopathy Associated with Long-Term Pentosan Polysulfate Therapy. Ophthalmology. Jun 2020;127(6):835-836. doi:10.1016/j.ophtha.2020.01.017 DOI: https://doi.org/10.1016/j.ophtha.2020.01.017
Wang D, Au A, Gunnemann F, et al. Pentosan-associated maculopathy: prevalence, screening guidelines, and spectrum of findings based on prospective multimodal analysis. Can J Ophthalmol. Apr 2020;55(2):116-125. doi:10.1016/j.jcjo.2019.12.001 DOI: https://doi.org/10.1016/j.jcjo.2019.12.001
Higgins K, Welch RJ, Bacorn C, et al. Identification of Patients with Pentosan Polysulfate Sodium-Associated Maculopathy through Screening of the Electronic Medical Record at an Academic Center. J Ophthalmol. 2020;2020:8866961. doi:10.1155/2020/8866961 DOI: https://doi.org/10.1155/2020/8866961
Philip AM, Wannamaker KW, Miller DM. Prevalence and Dose Dependency Analysis of Pentosan Polysulfate Sodium Maculopathy. Ophthalmic Epidemiol. Jan 26 2022:1-6. doi:10.1080/09286586.2022.2031227 DOI: https://doi.org/10.1080/09286586.2022.2031227
Kalbag NS, Maganti N, Lyon AT, Mirza RG. Maculopathy Secondary to Pentosan Polysulfate Use: A Single-Center Experience. Clin Ophthalmol. 2021;15:513-519. doi:10.2147/opth.S285013 DOI: https://doi.org/10.2147/OPTH.S285013
Leung EH, Sharma S, Levie-Sprick A, Lee GD, Cho H, Mukkamala K. Pentosan Polysulfate Sodium-Associated Pigmentary Retinopathy: Risk Factors and Fundus Findings. Clin Ophthalmol. 2021;15:4809-4816. doi:10.2147/opth.S340041 DOI: https://doi.org/10.2147/OPTH.S340041
Jain N, Li AL, Yu Y, VanderBeek BL. Association of macular disease with long-term use of pentosan polysulfate sodium: findings from a US cohort. Br J Ophthalmol. Aug 2020;104(8):1093-1097. doi:10.1136/bjophthalmol-2019-314765 DOI: https://doi.org/10.1136/bjophthalmol-2019-314765
Ludwig CA, Vail D, Callaway NF, Pasricha MV, Moshfeghi DM. Pentosan Polysulfate Sodium Exposure and Drug-Induced Maculopathy in Commercially Insured Patients in the United States. Ophthalmology. Apr 2020;127(4):535-543. doi:10.1016/j.ophtha.2019.10.036 DOI: https://doi.org/10.1016/j.ophtha.2019.10.036
VanderBeek BL, Jain N. Re: Ludwig et al.: Pentosan polysulfate sodium exposure and drug-induced maculopathy in commercially insured patients in the United States (Ophthalmology. 2020;127:535-543). Ophthalmology. May 2020;127(5):e35-e36. doi:10.1016/j.ophtha.2020.01.004 DOI: https://doi.org/10.1016/j.ophtha.2020.01.004
Jain N, Liao A, Garg SJ, et al. Expanded Clinical Spectrum of Pentosan Polysulfate Maculopathy: A Macula Society Collaborative Study. Ophthalmol Retina. Mar 2022;6(3):219-227. doi:10.1016/j.oret.2021.07.004 DOI: https://doi.org/10.1016/j.oret.2021.07.004
Lyons RJ, Brower J, Jain N. Visual Function in Pentosan Polysulfate Sodium Maculopathy. Invest Ophthalmol Vis Sci. Nov 2 2020;61(13):33. doi:10.1167/iovs.61.13.33 DOI: https://doi.org/10.1167/iovs.61.13.33
Hanif AM, Armenti ST, Taylor SC, et al. Phenotypic Spectrum of Pentosan Polysulfate Sodium-Associated Maculopathy: A Multicenter Study. JAMA Ophthalmol. Nov 1 2019;137(11):1275-1282. doi:10.1001/jamaophthalmol.2019.3392 DOI: https://doi.org/10.1001/jamaophthalmol.2019.3392
Barnes AC, Hanif AM, Jain N. Pentosan Polysulfate Maculopathy versus Inherited Macular Dystrophies: Comparative Assessment with Multimodal Imaging. Ophthalmol Retina. Dec 2020;4(12):1196-1201. doi:10.1016/j.oret.2020.05.008 DOI: https://doi.org/10.1016/j.oret.2020.05.008
Christiansen JS, Barnes AC, Berry DE, Jain N. Pentosan polysulfate maculopathy versus age-related macular degeneration: comparative assessment with multimodal imaging. Can J Ophthalmol. Feb 2022;57(1):16-22. doi:10.1016/j.jcjo.2021.02.007 DOI: https://doi.org/10.1016/j.jcjo.2021.02.007
Mishra K, Patel TP, Singh MS. Choroidal Neovascularization Associated with Pentosan Polysulfate Toxicity. Ophthalmol Retina. Jan 2020;4(1):111-113. doi:10.1016/j.oret.2019.08.006 DOI: https://doi.org/10.1016/j.oret.2019.08.006
Abou-Jaoude MM, Davis AM, Fraser CE, et al. New Insights Into Pentosan Polysulfate Maculopathy. Ophthalmic Surg Lasers Imaging Retina. Jan 1 2021;52(1):13-22. doi:10.3928/23258160-20201223-04 DOI: https://doi.org/10.3928/23258160-20201223-04
De Larochellière E, Bourgault S. Pentosan polysulfate sodium-induced pigmentary maculopathy with non-leaking cystoid macular edema successfully treated with anti-VEGF therapy. Retin Cases Brief Rep. Jun 5 2020. doi:10.1097/icb.0000000000001013 DOI: https://doi.org/10.1097/ICB.0000000000001013
Shah R, Simonett JM, Lyons RJ, Rao RC, Pennesi ME, Jain N. Disease Course in Patients With Pentosan Polysulfate Sodium-Associated Maculopathy After Drug Cessation. JAMA Ophthalmol. Aug 1 2020;138(8):894-900. doi:10.1001/jamaophthalmol.2020.2349 DOI: https://doi.org/10.1001/jamaophthalmol.2020.2349
Wang J, Ying GS. Growth Rate of Geographic Atrophy Secondary to Age-Related Macular Degeneration: A Meta-Analysis of Natural History Studies and Implications for Designing Future Trials. Ophthalmic Research. 2021;64(2):205-215. doi:10.1159/000510507 DOI: https://doi.org/10.1159/000510507
Barnett JM, Jain N. Potential new onset clinically detectable pentosan polysulfate maculopathy years after drug cessation. Retin Cases Brief Rep. Nov 17 2020. doi:10.1097/icb.0000000000001090 DOI: https://doi.org/10.1097/ICB.0000000000001090
Huckfeldt RM, Vavvas DG. Progressive Maculopathy After Discontinuation of Pentosan Polysulfate Sodium. Ophthalmic Surg Lasers Imaging Retina. Oct 1 2019;50(10):656-659. doi:10.3928/23258160-20191009-10 DOI: https://doi.org/10.3928/23258160-20191009-10
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